Rabbit anti-Human MCCC2 Polyclonal Antibody | anti-MCCC2 antibody

Anti-MCCC2 Antibody, Rabbit Polyclonal

Cat. Num. AAA8108115

• Reactivity: Human
• Applications: Immunohistochemistry, Immunocytochemistry, Immunofluorescence
• Purity: Protein A & Antigen Affinity
• Synonyms: MCCC2; Polyclonal Antibody; Anti-MCCC2 Antibody; Rabbit Polyclonal; MCCC2 Antibody; Rabbit PAb; Antigen Affinity Purified; methylcrotonoyl-CoA carboxylase 2 (beta); Anti-MCCB Antibody; anti-MCCC2 antibody

• Host: Rabbit
• Reactivity: Human
• Clonality: Polyclonal
• Isotype: Rabbit IgG
• Specificity: Human MCCC2
• Purity/Purification: Protein A & Antigen Affinity
• Form/Format: Liquid; PBS, pH7.0 with 0.03% Proclin300

• Applicable Applications for anti-MCCC2 antibody: Immunohistochemistry-Paraffin (IHC-P), Immunocytochemistry (ICC), Immunofluorescence (IF)
• Application Notes: IHC-P: 1:50-1:200; ICC/IF: 1:50-1:200
• Immunogen: E Coli-derived Human MCCC2 fragment
• Conjugation: Unconjugated
• Preparation: Produced in rabbits immunized with E Coli-derived Human MCCC2 fragment, and purified by antigen affinity chromatography.
• Preparation and Storage: This antibody can be stored at 2-8 degree C for one month without detectable loss of activity. Antibody products are stable for twelve months from date of receipt when stored at -20 degree C to -80 degree C. Avoid repeated freeze-thaw cycles.

Immunohistochemistry (IHC)

(Immunochemical staining of human MCCC2 in human kidney with rabbit polyclonal antibody at 1:100 dilution, formalin-fixed paraffin embedded sections.)

Immunofluorescence (IF)

(Immunofluorescence staining of MCCC2 in A431 cells. Cells were fixed with 4% PFA, permeabilzed with 0.1% Triton X-100 in PBS,blocked with 10% serum, and incubated with rabbit anti-Human MCCC2 polyclonal antibody (dilution ratio 1:100) at 4 degree C overnight. Then cells were stained with the Alexa Fluor488-conjugated Goat Anti-rabbit IgG secondary antibody (green) and counterstained with DAPI (blue).Positive staining was localized to Cytoplasm.)

Related Product Information for anti-MCCC2 antibody

3-methylcrotonyl-CoA carboxylase (MCC) deficiency is an autosomal recessive disorder of leucine metabolism caused by mutations in MCCC1 or MCCC2 encoding the alpha and beta subunit of MCC, respectively. Methylcrotonoyl-CoA carboxylase 2 (MCCC2), a subunit of 3-Methylcrotonyl-CoA carboxylase (MCC), is involved in tumor formation and development. MCCC1 and MCCC2 were expressed at high levels in muscle and ovary, but only trace levels in the heart, spleen, and liver. MCCase appears to be particularly important in muscle and ovary, which are active in protein metabolism, while PCase is important in organs active in glycolysis, such as the liver. Mutations on MCCC1 and MCCC2 genes are the major genetic causes for the increased C5-OH in neonates, and maternal single heterozygous mutation can contribute to the moderately to severely increased C5-OH.

References

Wu D, et al. (2019) [genetic analysis of newborns with abnormal metabolism of 3-hydroxyisovalerylcarnitine]. Zhejiang Da Xue Xue Bao Yi Xue Ban 48 (4): 390-396.Liu Y, et al. (2019) Methylcrotonoyl-coa carboxylase 2 overexpression predicts an unfavorable prognosis and promotes cell proliferation in breast cancer. Biomark Med 13 (6): 427-436.Fonseca H, et al. (2016) 3-methylcrotonyl-coa carboxylase deficiency: Mutational spectrum derived from comprehensive newborn screening. Gene 594 (2): 203-210.Abe S, et al. (2004) Isolation and identification of 3-methylcrotonyl coenzyme a carboxylase cdnas and pyruvate carboxylase, and their expression in red seabream (pagrus major) organs. Mar Biotechnol (NY) 6 (6): 527-540.